QD clinics on ILD - lessons from the Clinic #3 Save
QD clinics on ILD - lessons from the Clinic; brought to during the "Rheum to Breathe" ILD campaign
ILD QD Clinic #3
ILD QD Clinic: Application of the ACR CHEST Guidelines to Two Cases
ILD QD Clinic: Progressive RA-ILD Management
ILD QD Clinic: Beyond the Numbers in Newly Diagnosed ILD
Transcription
Hello, my name is Doctor. Sindhu Johnson, and I'm a professor of medicine at the University of Toronto. I would like to thank RheumNow for the opportunity to share with you application of the ACR chest guidelines for the screening, monitoring and treatment of scleroderma associated ILD, as well as rapidly progressive ILD. Case one. A 55 year old male is referred to you for evaluation of systemic sclerosis.
He has Raynaud phenomenon since 2019, puffy fingers since 2023, and symptoms of gastroesophageal reflux disease, joint pain, poor sleep, pruritus, fatigue, and mild shortness of breath. His blood work is fairly unremarkable, with the exception of elevated ESR and elevated CRP and positive SCL 70 antibodies. On physical exam, his lungs are clear to auscultation, however, his modified Rodin Skin score is 23 out of 51, with flexion contractures of his hands and skin thickening of his hands, arms, legs, and face. When we apply the American College of Rheumatology American College of Test Physician guidelines, there is a conditional recommendation to screen with pulmonary function tests spirometry, lung volumes, diffusion capacity, as well as high resolution CT chest. We performed these tests and found that his FVC was 77% predicted, TLC was 73% predicted, and his DLCO was 73% predicted.
A CT chest demonstrated by basilar fibrosis. When we consider his risk for not only having ILD but also progression of ILD, he has a number of risk factors. He's SCL seventy positive, he has a diffuse subtype, he's male, and he has early disease with acute phrase reactants. So not only can we make a diagnosis of interstitial lung disease, but because he has shortness of breath, radiographic findings, and risk factors, one would choose to treat his interstitial lung disease. In this case, the guidelines have a menu of options that we can choose from: mycophenolate, which is the first line treatment, but also one could consider tocilizumab, rituximab, cyclophosphamide, nintanenib, and azathioprine.
There is a strong recommendation against the use of glucocorticoids due to the risk of inducing a scleroderma renal crisis. So in his case, he was prescribed mycophenolate mofetil at one thousand mg twice a day. The next grouping of recommendations relates to monitoring, and so it is recommended that in people with systemic sclerosis, they undergo pulmonary function testing every three to six months in the first year and then less frequently once stable. In addition, it is conditionally recommended that they undergo ambulatory saturation testing every three to twelve months, as well as high resolution CT chest. On follow-up, his mycophenolate one thousand milligrams twice a day is continued.
He denies fatigue, and pruritus has improved. However, he complains of shortness of breath with one flight of stairs and now a mild cough. He continues to have skin thickening with no improvement. His repeat pulmonary function tests show a reduction in FVC to 69% predicted and his DLCO at 66% predicted. Again, repeat pulmonary function testing shows further decline in his FVC to 67% as well as his DLCO to 65%.
His ESR is normal, but his CRP is elevated at 1.12, and his HAC DI is 1.5. Repeat CT scan of his chest demonstrates worsening fibrosis now migrating to the mid zones. In this case, we can say that he has progression of his ILD despite mycophenolate therapy. So the guidelines present another menu of options for those who have progression despite first line therapy. These include mycophenolate, if mycophenolate was not the first line therapy, rituximab, nintedinib, tocilizumab, cyclophosphamide, referral to and for consideration of autologous hematopoietic stem cell transplant.
There remains a strong recommendation against long term glucocorticoids in these patients. And at this point, one may consider referral to for lung transplant evaluation. In his case, he was treated with nintedinib. However, an equally reasonable option would be to switch or add tocilizumab or switch or add rituximab. Case two, a 63 year old male with antisynthetase syndrome.
He was diagnosed with idiopathic inflammatory myositis in 2020 when he presented with symptoms of muscle pain, fatigue, shortness of breath on exertion, and cough. He had evidence of mechanic's hands, proximal muscle weakness in his upper and lower extremities. His CK was elevated at 2,800. His EMG demonstrated fibrillation potentials, and his MRI with STIR imaging revealed muscle edema and inflammation. His serology was positive for JO-one antibody.
Based on the recommendations, he was screened for ILD and underwent pulmonary function testing, which revealed an FVC of 71%, TLC of 77%, and a DLCO of 67%. His CT chest revealed bibasilar fibrosis. In this case, first line treatment of myositis ILD includes mycophenolate, azathioprine, rituximab, a calcineurin inhibitor, JAK inhibitors, cyclophosphamide, and short term glucocorticoids. So in his case, he was started on mycophenolate five hundred BID as well as prednisone sixty milligrams a day. The mycophenolate was increased to a maintenance dose of one thousand milligrams twice a day while his prednisone was tapered over ten months.
However, in 2021, he comes back to clinic with a nonproductive cough of three months and slow worsening of his shortness of breath over two months. His repeat pulmonary function tests show a decline in his FVC to 56%, a decline in his DLCO to 46%, and although there was an improvement initially in his CK to 400, it has now increased to 840. In this case, where we have progression of ILD in someone who has myositis despite treatment of mycophenolate, there again is a menu of options that one can choose from. These include rituximab, a calcineurin inhibitor, nintedanib, cyclophosphamide, IVIG and JAK inhibitors. While one could consider short term steroids, there is a conditional recommendation against long term steroids.
The plan for him was to repeat the high resolution CT chest, rule out infection, and consider addition of moderate doses of prednisone and rituximab. However, a month later, he comes back to clinic with a sudden and significant worsening in shortness of breath in the past seven days. He's unable to walk even a few steps without shortness of breath on exertion, and so he went to the emergency room. There, they do not identify fever, but he is tachypneic. His oxygen saturation is 88%, requiring three liters of, binasal prongs.
His EKG shows sinus tachycardia with no other abnormalities, and they rule out pulmonary embolism. Repeat CT chest reveals marked worsening of his interstitial lung disease. In this case, we can make a diagnosis of rapidly progressive ILD. That is a subpopulation of those who have ILD that is characterized by rapid progression from no oxygen to a or baseline level of oxygen to high oxygen requirements or intubation occurring over days to weeks without documented alternative cause such as infection or heart failure. In this case, the guidelines conditionally recommend combination therapy with IV glucocorticoids plus two additional therapies.
In his case, he was treated with pulse steroids and rituximab one thousand milligrams two weeks apart, that is two doses, and he was continued on his mycophenolate a thousand milligrams twice daily. He was discharged from hospital on sixty milligrams of prednisone that was tapered over twelve months. And as you can see from this table, there was a progressive improvement in his forced vital capacity. This percent predicted from a low of 54% up to 70%, an improvement in his DLCO from 32% to 58%, and an improvement in his CK from 840 to 54. So I have tried to demonstrate for you the application of the ACR and CHEST guidelines.
These guidelines provide a risk based approach for the screening and monitoring for SSC ILD and rapidly progressive ILD. These guidelines provide a range of treatment options based on the best published evidence, expert experience, as well as patients' preferences and values. I'd like to thank everyone who was involved with the development of these guidelines. And finally, thank you for your attention.
Hello, everyone. I'm Richard Conway from Dublin, Ireland, and this is QD Clinic from RheumNow. And today's case is of progressive rheumatoid arthritis interstitial lung disease. So this is the case of a 58 year old man, He's an ex smoker. He's been attending my clinic for the last ten years or so.
He's seropositive, strongly so, for rheumatoid factor and CCP antibodies. And he has a known diagnosis of rheumatoid arthritis interstitial lung disease. He's on methotrexate and adalimumab for the treatment of his rheumatoid. His arthritis itself has been under relatively good control, seems to become a little more active in recent times. He's quite a stoic man, so he's not really complaining very much.
But when we do his disease activity measurements, his CDAI is 15, his DAS28 CRP is 3.3. So we're looking at somebody with a moderate level of disease activity. Seems acceptable to him, but possibly shouldn't be for us even in the absence of the interstitial lung disease. This man has some pulmonary symptoms. He's had a dry cough.
He doesn't have any dyspnea, but this cough is bothering him and interfering with his social activities more than anything. Especially in the modern post COVID era, people can get a bit self conscious about their coughs. We listen to his lungs, his crackles kind of bases up to the mid zones on both sides. So this is something we need to investigate further. We do a chest x-ray.
We see some fibrotic changes on that. We get him some pulmonary function tests. The pulmonary function tests show that he has an FVC of 57% and a DLCO of 45%. So straight off the bat, these are not good numbers. These are relatively severe and impaired pulmonary function tests for somebody.
She never looked at pulmonary function tests in isolation. So we know this man is interstitial only. So he's been attending our clinic. We've been keeping an eye on his pulmonary function tests. We look back on those as DLCO, now it's forty five percent.
A year ago, it was fifty five. A year before that, it was sixty five. So we've had quite a steady downward trajectory in this. And his FVC, it's fifty seven percent now. A year ago, it was sixty five percent.
Before that, it was seventy percent. So this man does seem to have progressive interstitial lung disease in the setting of rheumatoid arthritis. And the question is, what should we do about this? So he's on methotrexate and a TNF inhibitor. These had previously kept his disease under control.
They're not currently doing it. His rheumatoid is active and his interstitial lung disease is now progressing. And it's progressing down to relatively low levels at which we might start to be running into trouble in terms of the need for oxygen and possibly even considering things like lung transplants. So if we can do anything to arrest this disease process, we should do it now. And what should we do is the question.
And that is something that has been very controversial in recent times. There are options here. And maybe we should do one thing. Maybe we should do more than one thing. Different people might do different things in this situation.
What I'm thinking and what I have done here is that this man is on methotrexate. Should we continue that? And that's an easy answer for me. We should continue it. There's no reason to stop methotrexate here.
We're not dealing with pneumonitis worsening his lung function. We're dealing with progression of his long term rheumatoid pulmonary fibrosis interstitial lung disease process. And there's no evidence that methotrexate worsens that. There is some evidence that it might benefit it. So the only likely thing you are to achieve by stopping methotrexate is to make this man worse.
I don't think there's any possibility that that will make him better. We should do a CT scan to see exactly what's going on here because we know the lung disease is worsening, but what does it look like? So we did a CT scan in this man and we saw that he has basal, subpleural predominant fibrotic changes with honeycomann traction bronchiectasis. So he also had quite a bit of ground glass change throughout his lungs. This is suggesting that there is some reversibility to this process.
So our next consideration was with regard to his biologic agent. So the TNF inhibitor does not seem to be controlling either aspect of his disease process. So we would consider a change in that, and we did. In this setting, I changed this TNF inhibitor rituximab, is one of my favourite agents in this setting. Again, the evidence here is not necessarily fantastic, but I think it does have perhaps the best evidence in terms of biologic agents for treating this.
Other reasonable options would be pretty much any non TNF inhibitor biologics. So abatacept has some decent evidence, tocilizumab has some evidence, JAK inhibitors some emerging evidence as well. I think really the key is to change a treatment that's not working. In this case, we chose rituximab. We did a second thing here.
We added in nintedinib. And I know that might be controversial to some, but Nintedinib actually probably has our best level of evidence that is beneficial in rheumatoid arthritis interstitial lung disease, randomized control trial evidence. It has what some may argue is a mild to moderate benefit, but it is definitely a real benefit in slowing down the progression of the fibrotic process. Entatinib and agents like us, they do have quite high adverse event burden. So it's always something we're initiating as a trial of an agent, not necessarily something that they're definitely gonna stay on, but it's mantolerated well.
And so why not have it there for the benefits it can do in slowing down the progression of his ILD? So that's what I did in this situation, rituximab and nintedinib. Other people here might have either kept the TNF inhibitor there and added in mycophenolate mofetil or switched the TNF inhibitor to mycophenolate mofetil. It's not something I'm terribly comfortable doing when his arthritis disease process is also active. But someone advocate for doing that for his lung disease.
But I didn't. Rituximab and entetinib, we started these and we stopped the CnF inhibitor, continued the methotrexate. We saw him back again six months later and he was feeling a bit better. The cough was still there, but it had reduced. We did his pulmonary function tests again.
His DLCO was written back up to 55%. His FVC had improved back 63%. So things seemed to, they certainly stopped your downward progression. They seemed to be improving. So it seemed like we'd gotten this man back on track.
And his arthritic disease was now completely quiescent. So I think the learning point here is that if things aren't working out and they're progressing on your current treatment, you should change that to something else. There is limited evidence about what we should change it to. But the basic principle for me always is your initial priority is to achieve disease control of his rheumatoid arthritis process with whatever agents you need to do that. So that's been today's QD clinic and tune to RheumNow for more QD clinics.
Hello, welcome to QD clinic. My name is Doctor. Eric Dive. I'm a rheumatologist with Atlantic Health in Summit, New Jersey. And today, I'm going to talk about a case beyond the numbers of interstitial lung disease that came into my clinic just the other day.
She was a patient who was treated in an outside rheumatology clinic, came in for a new diagnosis of interstitial lung disease, presented initially with dyspnea and cough as her presenting symptoms. She denied any other symptoms, arthralgias, morning stiffness, rashes, muscle weakness, SICA, Raynaud's, sclerodactyly, you know, the whole review of systems we all know very well. Her physical examination was notable for bibasilar rales on pulmonary auscultation, but without any other rheumatic findings suggestive of RA lupus scleroderma or myositis. She had a workup done by her initial rheumatologist, which showed a very high positive SSA antibody, negative SSP antibody, and she was started on mycophenolate fifteen hundred milligrams twice daily. She came in for reevaluation because she was uncertain about this diagnosis of Sjogren's disease and was very poorly tolerating the mycophenolate, had a lot of GI symptoms with it.
And she was highly concerned about the idea of being on immunosuppressive therapy, given the infectious risks, particularly in light of having concomitant lung disease. And she had done some reading on Sjogren's disease, she said she really didn't have any sicker symptoms to speak of. So, I asked her all sorts of different questions about sicker. Does she have any dryness symptoms, gritty eye sensation, sand in the eye type of sensation? Could she eat a cracker without drinking water?
And she didn't have many symptoms of that. On reviewing her imaging, she had interstitial lung disease with a UIP pattern. It's worth noting that UIP pattern is more associated with the idiopathic pulmonary fibrosis, whereas NSIP is more closely associated with the autoimmune diseases. But that said, autoimmune etiology should certainly be highly considered for both. As an aside, the other interstitial lung disease to think about is lymphoid interstitial pneumonia, which is a rare form of ILD, but it is very associated with Sjogren's disease.
She did not have that, so she fit that UIP pattern. We discussed that not all patients with Sjogren's disease have Sika symptoms. Some have pretty significant Sika on objective testing without any symptoms of it. And many patients with Sjogren's do not have any dryness at all on workup. We did an objective test.
We did the Schirmer's test in the office. We also did a tear breakup study with her ophthalmologist. Those tests were normal. We thought that she'd be an excellent candidate to get a lip biopsy of her salivary gland to assess for Sjogren's disease via a gold standard technique. And she had a focus score of zero, so it did not show any evidence of Sjogren's disease.
So despite the SSA antibody, she really didn't have any other symptoms, other examination findings, or other pathologic findings to confirm a diagnosis of Sjogren's. When we see the SSA antibody, also think about, could this go along with lupus diagnosis? Could it go along with myositis diagnosis? She didn't have any features of lupus, had a negative ANA, had no objective weakness, had a normal CK. So, not all patients with positive serologies necessarily have an associated autoimmune diagnosis.
Given the ILD, and we see these CT undifferentiated cases that are associated with ILD, you would definitely want to have a high suspicion. But given her ILD and serologic findings, would want to watch her closely. But she had a conversation with us and we elected to stop the mycophenolate and we started her on anti fibrotic therapy. She had much better GI tolerance with the anti fibrotic therapy that she did with mycophenolate. And with pulmonary on board and with careful watching of her PFTs and CT scans, she has done really, really well.
So she's had stable findings on her symptoms and her objective testing on the anti fibrotic. So this is a patient who we've been able to keep off of immunosuppressive therapy and her indication remains unclear. So my takeaways and learning from this case is to always treat the patient, not necessarily the numbers. In rheumatology, very few lab findings are in of itself diagnostic of autoimmune disease in isolation, so always correlating back to the patient. Considering the exact phenotype of different types of ILD to help guide your management.
Not all patients have Sicka in Sjogren's disease, and not all patients with Sjogren's disease have sicka. But in times of uncertainty, remembering that there's the diagnostic testing to evaluate for sicka, and then there's also the salivary lip biopsy to get more information, more confirmation. And always reevaluate. So reevaluate both your own diagnoses and those made by prior providers and not to anchor upon one piece of information. So reassess the need for therapies when they're on it.
Upon discontinuing, especially always follow and consider if you need to reinstitute that therapy. Hope this was a helpful case and pay attention to RheumNow for lots more on ILD this month.
He has Raynaud phenomenon since 2019, puffy fingers since 2023, and symptoms of gastroesophageal reflux disease, joint pain, poor sleep, pruritus, fatigue, and mild shortness of breath. His blood work is fairly unremarkable, with the exception of elevated ESR and elevated CRP and positive SCL 70 antibodies. On physical exam, his lungs are clear to auscultation, however, his modified Rodin Skin score is 23 out of 51, with flexion contractures of his hands and skin thickening of his hands, arms, legs, and face. When we apply the American College of Rheumatology American College of Test Physician guidelines, there is a conditional recommendation to screen with pulmonary function tests spirometry, lung volumes, diffusion capacity, as well as high resolution CT chest. We performed these tests and found that his FVC was 77% predicted, TLC was 73% predicted, and his DLCO was 73% predicted.
A CT chest demonstrated by basilar fibrosis. When we consider his risk for not only having ILD but also progression of ILD, he has a number of risk factors. He's SCL seventy positive, he has a diffuse subtype, he's male, and he has early disease with acute phrase reactants. So not only can we make a diagnosis of interstitial lung disease, but because he has shortness of breath, radiographic findings, and risk factors, one would choose to treat his interstitial lung disease. In this case, the guidelines have a menu of options that we can choose from: mycophenolate, which is the first line treatment, but also one could consider tocilizumab, rituximab, cyclophosphamide, nintanenib, and azathioprine.
There is a strong recommendation against the use of glucocorticoids due to the risk of inducing a scleroderma renal crisis. So in his case, he was prescribed mycophenolate mofetil at one thousand mg twice a day. The next grouping of recommendations relates to monitoring, and so it is recommended that in people with systemic sclerosis, they undergo pulmonary function testing every three to six months in the first year and then less frequently once stable. In addition, it is conditionally recommended that they undergo ambulatory saturation testing every three to twelve months, as well as high resolution CT chest. On follow-up, his mycophenolate one thousand milligrams twice a day is continued.
He denies fatigue, and pruritus has improved. However, he complains of shortness of breath with one flight of stairs and now a mild cough. He continues to have skin thickening with no improvement. His repeat pulmonary function tests show a reduction in FVC to 69% predicted and his DLCO at 66% predicted. Again, repeat pulmonary function testing shows further decline in his FVC to 67% as well as his DLCO to 65%.
His ESR is normal, but his CRP is elevated at 1.12, and his HAC DI is 1.5. Repeat CT scan of his chest demonstrates worsening fibrosis now migrating to the mid zones. In this case, we can say that he has progression of his ILD despite mycophenolate therapy. So the guidelines present another menu of options for those who have progression despite first line therapy. These include mycophenolate, if mycophenolate was not the first line therapy, rituximab, nintedinib, tocilizumab, cyclophosphamide, referral to and for consideration of autologous hematopoietic stem cell transplant.
There remains a strong recommendation against long term glucocorticoids in these patients. And at this point, one may consider referral to for lung transplant evaluation. In his case, he was treated with nintedinib. However, an equally reasonable option would be to switch or add tocilizumab or switch or add rituximab. Case two, a 63 year old male with antisynthetase syndrome.
He was diagnosed with idiopathic inflammatory myositis in 2020 when he presented with symptoms of muscle pain, fatigue, shortness of breath on exertion, and cough. He had evidence of mechanic's hands, proximal muscle weakness in his upper and lower extremities. His CK was elevated at 2,800. His EMG demonstrated fibrillation potentials, and his MRI with STIR imaging revealed muscle edema and inflammation. His serology was positive for JO-one antibody.
Based on the recommendations, he was screened for ILD and underwent pulmonary function testing, which revealed an FVC of 71%, TLC of 77%, and a DLCO of 67%. His CT chest revealed bibasilar fibrosis. In this case, first line treatment of myositis ILD includes mycophenolate, azathioprine, rituximab, a calcineurin inhibitor, JAK inhibitors, cyclophosphamide, and short term glucocorticoids. So in his case, he was started on mycophenolate five hundred BID as well as prednisone sixty milligrams a day. The mycophenolate was increased to a maintenance dose of one thousand milligrams twice a day while his prednisone was tapered over ten months.
However, in 2021, he comes back to clinic with a nonproductive cough of three months and slow worsening of his shortness of breath over two months. His repeat pulmonary function tests show a decline in his FVC to 56%, a decline in his DLCO to 46%, and although there was an improvement initially in his CK to 400, it has now increased to 840. In this case, where we have progression of ILD in someone who has myositis despite treatment of mycophenolate, there again is a menu of options that one can choose from. These include rituximab, a calcineurin inhibitor, nintedanib, cyclophosphamide, IVIG and JAK inhibitors. While one could consider short term steroids, there is a conditional recommendation against long term steroids.
The plan for him was to repeat the high resolution CT chest, rule out infection, and consider addition of moderate doses of prednisone and rituximab. However, a month later, he comes back to clinic with a sudden and significant worsening in shortness of breath in the past seven days. He's unable to walk even a few steps without shortness of breath on exertion, and so he went to the emergency room. There, they do not identify fever, but he is tachypneic. His oxygen saturation is 88%, requiring three liters of, binasal prongs.
His EKG shows sinus tachycardia with no other abnormalities, and they rule out pulmonary embolism. Repeat CT chest reveals marked worsening of his interstitial lung disease. In this case, we can make a diagnosis of rapidly progressive ILD. That is a subpopulation of those who have ILD that is characterized by rapid progression from no oxygen to a or baseline level of oxygen to high oxygen requirements or intubation occurring over days to weeks without documented alternative cause such as infection or heart failure. In this case, the guidelines conditionally recommend combination therapy with IV glucocorticoids plus two additional therapies.
In his case, he was treated with pulse steroids and rituximab one thousand milligrams two weeks apart, that is two doses, and he was continued on his mycophenolate a thousand milligrams twice daily. He was discharged from hospital on sixty milligrams of prednisone that was tapered over twelve months. And as you can see from this table, there was a progressive improvement in his forced vital capacity. This percent predicted from a low of 54% up to 70%, an improvement in his DLCO from 32% to 58%, and an improvement in his CK from 840 to 54. So I have tried to demonstrate for you the application of the ACR and CHEST guidelines.
These guidelines provide a risk based approach for the screening and monitoring for SSC ILD and rapidly progressive ILD. These guidelines provide a range of treatment options based on the best published evidence, expert experience, as well as patients' preferences and values. I'd like to thank everyone who was involved with the development of these guidelines. And finally, thank you for your attention.
Hello, everyone. I'm Richard Conway from Dublin, Ireland, and this is QD Clinic from RheumNow. And today's case is of progressive rheumatoid arthritis interstitial lung disease. So this is the case of a 58 year old man, He's an ex smoker. He's been attending my clinic for the last ten years or so.
He's seropositive, strongly so, for rheumatoid factor and CCP antibodies. And he has a known diagnosis of rheumatoid arthritis interstitial lung disease. He's on methotrexate and adalimumab for the treatment of his rheumatoid. His arthritis itself has been under relatively good control, seems to become a little more active in recent times. He's quite a stoic man, so he's not really complaining very much.
But when we do his disease activity measurements, his CDAI is 15, his DAS28 CRP is 3.3. So we're looking at somebody with a moderate level of disease activity. Seems acceptable to him, but possibly shouldn't be for us even in the absence of the interstitial lung disease. This man has some pulmonary symptoms. He's had a dry cough.
He doesn't have any dyspnea, but this cough is bothering him and interfering with his social activities more than anything. Especially in the modern post COVID era, people can get a bit self conscious about their coughs. We listen to his lungs, his crackles kind of bases up to the mid zones on both sides. So this is something we need to investigate further. We do a chest x-ray.
We see some fibrotic changes on that. We get him some pulmonary function tests. The pulmonary function tests show that he has an FVC of 57% and a DLCO of 45%. So straight off the bat, these are not good numbers. These are relatively severe and impaired pulmonary function tests for somebody.
She never looked at pulmonary function tests in isolation. So we know this man is interstitial only. So he's been attending our clinic. We've been keeping an eye on his pulmonary function tests. We look back on those as DLCO, now it's forty five percent.
A year ago, it was fifty five. A year before that, it was sixty five. So we've had quite a steady downward trajectory in this. And his FVC, it's fifty seven percent now. A year ago, it was sixty five percent.
Before that, it was seventy percent. So this man does seem to have progressive interstitial lung disease in the setting of rheumatoid arthritis. And the question is, what should we do about this? So he's on methotrexate and a TNF inhibitor. These had previously kept his disease under control.
They're not currently doing it. His rheumatoid is active and his interstitial lung disease is now progressing. And it's progressing down to relatively low levels at which we might start to be running into trouble in terms of the need for oxygen and possibly even considering things like lung transplants. So if we can do anything to arrest this disease process, we should do it now. And what should we do is the question.
And that is something that has been very controversial in recent times. There are options here. And maybe we should do one thing. Maybe we should do more than one thing. Different people might do different things in this situation.
What I'm thinking and what I have done here is that this man is on methotrexate. Should we continue that? And that's an easy answer for me. We should continue it. There's no reason to stop methotrexate here.
We're not dealing with pneumonitis worsening his lung function. We're dealing with progression of his long term rheumatoid pulmonary fibrosis interstitial lung disease process. And there's no evidence that methotrexate worsens that. There is some evidence that it might benefit it. So the only likely thing you are to achieve by stopping methotrexate is to make this man worse.
I don't think there's any possibility that that will make him better. We should do a CT scan to see exactly what's going on here because we know the lung disease is worsening, but what does it look like? So we did a CT scan in this man and we saw that he has basal, subpleural predominant fibrotic changes with honeycomann traction bronchiectasis. So he also had quite a bit of ground glass change throughout his lungs. This is suggesting that there is some reversibility to this process.
So our next consideration was with regard to his biologic agent. So the TNF inhibitor does not seem to be controlling either aspect of his disease process. So we would consider a change in that, and we did. In this setting, I changed this TNF inhibitor rituximab, is one of my favourite agents in this setting. Again, the evidence here is not necessarily fantastic, but I think it does have perhaps the best evidence in terms of biologic agents for treating this.
Other reasonable options would be pretty much any non TNF inhibitor biologics. So abatacept has some decent evidence, tocilizumab has some evidence, JAK inhibitors some emerging evidence as well. I think really the key is to change a treatment that's not working. In this case, we chose rituximab. We did a second thing here.
We added in nintedinib. And I know that might be controversial to some, but Nintedinib actually probably has our best level of evidence that is beneficial in rheumatoid arthritis interstitial lung disease, randomized control trial evidence. It has what some may argue is a mild to moderate benefit, but it is definitely a real benefit in slowing down the progression of the fibrotic process. Entatinib and agents like us, they do have quite high adverse event burden. So it's always something we're initiating as a trial of an agent, not necessarily something that they're definitely gonna stay on, but it's mantolerated well.
And so why not have it there for the benefits it can do in slowing down the progression of his ILD? So that's what I did in this situation, rituximab and nintedinib. Other people here might have either kept the TNF inhibitor there and added in mycophenolate mofetil or switched the TNF inhibitor to mycophenolate mofetil. It's not something I'm terribly comfortable doing when his arthritis disease process is also active. But someone advocate for doing that for his lung disease.
But I didn't. Rituximab and entetinib, we started these and we stopped the CnF inhibitor, continued the methotrexate. We saw him back again six months later and he was feeling a bit better. The cough was still there, but it had reduced. We did his pulmonary function tests again.
His DLCO was written back up to 55%. His FVC had improved back 63%. So things seemed to, they certainly stopped your downward progression. They seemed to be improving. So it seemed like we'd gotten this man back on track.
And his arthritic disease was now completely quiescent. So I think the learning point here is that if things aren't working out and they're progressing on your current treatment, you should change that to something else. There is limited evidence about what we should change it to. But the basic principle for me always is your initial priority is to achieve disease control of his rheumatoid arthritis process with whatever agents you need to do that. So that's been today's QD clinic and tune to RheumNow for more QD clinics.
Hello, welcome to QD clinic. My name is Doctor. Eric Dive. I'm a rheumatologist with Atlantic Health in Summit, New Jersey. And today, I'm going to talk about a case beyond the numbers of interstitial lung disease that came into my clinic just the other day.
She was a patient who was treated in an outside rheumatology clinic, came in for a new diagnosis of interstitial lung disease, presented initially with dyspnea and cough as her presenting symptoms. She denied any other symptoms, arthralgias, morning stiffness, rashes, muscle weakness, SICA, Raynaud's, sclerodactyly, you know, the whole review of systems we all know very well. Her physical examination was notable for bibasilar rales on pulmonary auscultation, but without any other rheumatic findings suggestive of RA lupus scleroderma or myositis. She had a workup done by her initial rheumatologist, which showed a very high positive SSA antibody, negative SSP antibody, and she was started on mycophenolate fifteen hundred milligrams twice daily. She came in for reevaluation because she was uncertain about this diagnosis of Sjogren's disease and was very poorly tolerating the mycophenolate, had a lot of GI symptoms with it.
And she was highly concerned about the idea of being on immunosuppressive therapy, given the infectious risks, particularly in light of having concomitant lung disease. And she had done some reading on Sjogren's disease, she said she really didn't have any sicker symptoms to speak of. So, I asked her all sorts of different questions about sicker. Does she have any dryness symptoms, gritty eye sensation, sand in the eye type of sensation? Could she eat a cracker without drinking water?
And she didn't have many symptoms of that. On reviewing her imaging, she had interstitial lung disease with a UIP pattern. It's worth noting that UIP pattern is more associated with the idiopathic pulmonary fibrosis, whereas NSIP is more closely associated with the autoimmune diseases. But that said, autoimmune etiology should certainly be highly considered for both. As an aside, the other interstitial lung disease to think about is lymphoid interstitial pneumonia, which is a rare form of ILD, but it is very associated with Sjogren's disease.
She did not have that, so she fit that UIP pattern. We discussed that not all patients with Sjogren's disease have Sika symptoms. Some have pretty significant Sika on objective testing without any symptoms of it. And many patients with Sjogren's do not have any dryness at all on workup. We did an objective test.
We did the Schirmer's test in the office. We also did a tear breakup study with her ophthalmologist. Those tests were normal. We thought that she'd be an excellent candidate to get a lip biopsy of her salivary gland to assess for Sjogren's disease via a gold standard technique. And she had a focus score of zero, so it did not show any evidence of Sjogren's disease.
So despite the SSA antibody, she really didn't have any other symptoms, other examination findings, or other pathologic findings to confirm a diagnosis of Sjogren's. When we see the SSA antibody, also think about, could this go along with lupus diagnosis? Could it go along with myositis diagnosis? She didn't have any features of lupus, had a negative ANA, had no objective weakness, had a normal CK. So, not all patients with positive serologies necessarily have an associated autoimmune diagnosis.
Given the ILD, and we see these CT undifferentiated cases that are associated with ILD, you would definitely want to have a high suspicion. But given her ILD and serologic findings, would want to watch her closely. But she had a conversation with us and we elected to stop the mycophenolate and we started her on anti fibrotic therapy. She had much better GI tolerance with the anti fibrotic therapy that she did with mycophenolate. And with pulmonary on board and with careful watching of her PFTs and CT scans, she has done really, really well.
So she's had stable findings on her symptoms and her objective testing on the anti fibrotic. So this is a patient who we've been able to keep off of immunosuppressive therapy and her indication remains unclear. So my takeaways and learning from this case is to always treat the patient, not necessarily the numbers. In rheumatology, very few lab findings are in of itself diagnostic of autoimmune disease in isolation, so always correlating back to the patient. Considering the exact phenotype of different types of ILD to help guide your management.
Not all patients have Sicka in Sjogren's disease, and not all patients with Sjogren's disease have sicka. But in times of uncertainty, remembering that there's the diagnostic testing to evaluate for sicka, and then there's also the salivary lip biopsy to get more information, more confirmation. And always reevaluate. So reevaluate both your own diagnoses and those made by prior providers and not to anchor upon one piece of information. So reassess the need for therapies when they're on it.
Upon discontinuing, especially always follow and consider if you need to reinstitute that therapy. Hope this was a helpful case and pay attention to RheumNow for lots more on ILD this month.
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